Pdf dexamethasone treatment for congenital adrenal. The salt losing crisis in an infant with congenital adrenal hyperplasia requires treatment with in travenous normal saline, the amount being calcu. Congenital adrenal hyperplasia cah refers to a range of autosomal recessive diseases resulting from deficiency of cortisol secretion. Congenital adrenal hyperplasia in the newborn pdf minnesota. Guidelines for diagnosis and treatment of 21hydroxylase deficiency. Challenges in the treatment of congenital adrenal hyperplasia include avoidance of glucocorticoid overtreatment and control of sex hormone imbalances. Do the benefits of prenatal treatment defeat the risks. Prenatal dexamethasone for congenital adrenal hyperplasia. Congenital adrenal hyperplasia due to 21hydroxylase deficiency. Diagnosis and management of congenital adrenal hyperplasia. The only form of cah detected by kansas newborn screening is 21hydroxylase deficiency. The term congenital adrenal hyperplasia often shortened to cah describes the condition.
Congenital adrenal hyperplasia is the name applied to a class of autosomal recessive disorders resulting from deficiency of one of five enzymes necessary for cortisol synthesis by the adrenal cortex. Congenital adrenal hyperplasia guideline resources endocrine. Dexamethasone treatment for congenital adrenal hyperplasia article pdf available in archives of disease in childhood 653. Thus, we do not recommend specific treatment protocols. Patients with classic cah require treatment with glucocorticosteroids and, in cases of salt wasting, also with a mineralocorticosteroid. Management of congenital adrenal hyperplasia archives of. Clan is committed to working with cah families and their health professionals over the longer term, enacting the 5 pillars of clan focusing also on education, optimal medical management, support group networks and reducing poverty. Congenital adrenal hyperplasia american academy of pediatrics. Clinical symptoms the clinical phenotype depends on the nature and severity of the enzyme deficiency. Nonclassic congenital adrenal hyperplasia, international journal of pediatric endocrinology. Patients with congenital adrenal hyperplasia and parents of minors should seek mental health treatment to address any congenital adrenal. The doctor will monitor your child in order to know what dose of medicine is needed. Video demonstration of emergency hc intramuscular injection. Prenatal treatment of congenital adrenal hyperplasia 2.
Successful treatment requires teamwork between you and your doctor. Congenital adrenal hyperplasia due to steroid 21hydroxylase. Congenital adrenal hyperplasia journal of pediatric and. Prenatal treatment of congenital adrenal hyperplasia not standard of care. Indications for treatment and followup of individuals with nonclassic congenital adrenal hyperplasia due to 21hydroxylase deficiency. See more ideas about congenital adrenal hyperplasia. Longterm complications include abnormal growth and development, adverse effects on bone and the cardiovascular system, and infertility.
We recommend that prenatal treatment of cah continue to be regarded as. Pdf we developed clinical practice guidelines for congenital adrenal hyperplasia. The goal of treatment is normal growth and development as well as. Challenges in the treatment of congenital adrenal hyperplasia include avoidance of glucocorticoid overtreatment and control of sex hormone. Treatment of classic congenital adrenal hyperplasia. Diagnosis and management of the patient with nonclassic cah due.
Congenital adrenal hyperplasia cah information for. Labcorp test details for congenital adrenal hyperplasia cah pediatric profile seven. Congenital adrenal hyperplasia, then, is an inherited disorder that affects the production of certain hormones and causes the adrenal glands to become too big hyperplastic. Pdf congenital adrenal hyperplasia due to steroid 21.